Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy
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(2020) 2:42
Neurological Research and Practice
REVIEW
Open Access
Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy Anna Lena Fisse1,2†, Jeremias Motte1,2*† , Thomas Grüter1,2, Melissa Sgodzai1,2, Kalliopi Pitarokoili1,2 and Ralf Gold1,2
Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic inflammatory neuropathy. CIDP is diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria, which combine clinical features with the electrophysiological evidence of demyelination. However, firstly, diagnosis is challenging, as some patients e.g. with severe early axonal damage do not fulfil the criteria. Secondly, objective and reliable tools to monitor the disease course are lacking. Thirdly, about 25% of CIDP patients do not respond to evidence-based first-line therapy. Recognition of these patients is difficult and treatment beyond first-line therapy is based on observational studies and case series only. Individualized immunomodulatory treatment does not exist due to the lack of understanding of essential aspects of the underlying pathophysiology. Novel diagnostic imaging techniques and molecular approaches can help to solve these problems but do not find enough implementation. This review gives a comprehensive overview of novel diagnostic techniques and monitoring approaches for CIDP and how these can lead to individualized treatment and better understanding of pathophysiology. Keywords: Chronic inflammatory demyelinating polyneuropathy, Inflammatory neuropathies, Imaging, Pathophysiology, Diagnosis, Monitoring, Treatment, Register, Biobank
Background The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic inflammatory neuropathy. Chronic and recurrent polyneuritis was first described in 1890 by Eichhorst (Eichhorst H.: Polyneuritis recurrens. Correspondenzblatt f. Schweizer Ärzte 1890, publication not digitally available). Around 1950 reports about steroid responsive chronic polyneuritis arose. The term ‘chronic inflammatory demyelinating * Correspondence: [email protected] † Anna Lena Fisse and Jeremias Motte contributed equally to this work. 1 Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Gudrunstrasse 56, 44791 Bochum, Germany 2 Immunmediated Neuropathies Biobank (INHIBIT), Ruhr-University Bochum, Bochum, Germany
polyradiculoneuropathy’ was firstly described by Dyck et al. 1982 [1]. CIDP is a relapsing-remitting or progressive inflammatory neuropathy with a multifaceted presentation. There are multiple other chronic inflammatory neuropathies besides CIDP. In the past decades several diagnostic criteria for diagnosis of CIDP were established. The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria [2] published in 2006 and revised in 2010, were validated in a multicenter European cohort and have since been broadly adopted in special for
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